Google translation from the Dutch
Romanovs members of hemophilia B
03-11-2009 Femia Kievits
A genetic study of bone remains of the Russian imperial family shows that Crown Prince Alexei Romanov – and with it likely many of Queen Victoria’s other male descendants – suffered from haemophilia B (Science., Doi: 10.1126 / science.1180660).
It has long been known that haemophilia is common in royal descendants in this family. First, Leopold, son of the British Queen Victoria, fell ill. He bled to death from a knee injury in 1884 at the age of 31. Three male grandchildren suffered the same fate at ages 2, 23 and 32. Girls did not get sick, but some were carriers of the hereditary defects.
The most famous royal haemophiliac is Russian heir apparent Alexei Romanov, son of Nicholas II and Alexandra Feodorovna and great-grandchild of Queen Victoria. In his early childhood he showed an increased tendency to bleed, which some historians say has influenced the course of history. To cure his condition, his mother asked the advice of faith healer Grigori Rasputin. As a result, outrage grew among the Russian people about the influence of the royal family, which is seen as a part of the Russian revolution. Alexei did not die of his illness, he was executed by the Bolsheviks in 1918 with the rest of his family in Yekaterinburg.
In July 2007, bone remains were found at the crime scene, which Evgeny Rogaev et al. Demonstrated in early 2009 that they belonged to the Tsar’s family (PNAS., Doi: 10.1073 / pnas.0811190106). Now the researchers posthumously diagnose hemophilia B. Hemophilia B lacks coagulation factor IX. Although it was always assumed to be haemophilia A, no abnormalities were found in gene F8 – which codes for coagulation factor VIII. This was different with gene F9, where a mutation was found in exon 4 that, according to the researchers, is related to hemophilia B.
Rogaev et al. Also found that mother Alexandra was a carrier of the same gene mutation. Additional in vitro experiments suggest that in the Tsarevich, the production of coagulation factor IX was 99% defective. However, confirmation by living offspring is no longer possible because the last male offspring with the disease, Waldemar of Prussia, died in 1945.